Juvenile Idiopathic

Arthritis
Abdi M
 Evaluation of a Suspected Rheumatic Disease

• Rheumatic diseases are defined by the constellation of history , physical

examination, autoimmune marker and other serologic tests, tissue
pathology, and imaging.
• Considered in a wide range of differentials with no single confirmatory
diagnostic test.
• Most of rheumatologic conditions have their own diagnostic criteria.
Cont…
• Infection and malignancy but also include metabolic, orthopedic, immune
deficiencies, autoinflammatory diseases, and chronic pain conditions are
among the non rheumatologic differentials
• Overlap of clinical features is seen among different rheumatologic
illnesses.
• Some symptoms and signs although non-specific can help strengthen the
suspicion.
Cont…
• Arthralgia/Arthritis major feature in most rheumatologic conditions
(JIA,SLE)
• Morning stiffness Inflammatory Arthritis ,SLE
• Facial rash+joint complaints/weakness JDM,SLE
• Muscle weakness JDM, MCTD
• Persisting oral ulcers SLE
• Joint swelling JIA
Cont…
• Raynaud Phenomenon Scleroderma/SLE
• Fever SLE, systemic JIA,
• History of enteric illness Reactive arthritis
• Decreased mov’t CNS ds, skeletal abn,JDM
• Diffuse lymphadenopathy SLE, polyarticular JIA, and systemic
Pathogenesis
- Immune Tolerance
Immune Dysregulation - Polymorphism
- Cytokines

- MHC 1 & 2
Genetic Susceptibility - Non HLA loci
encoding TNF,IL-
1,6
- Family and Twin
studies
- Viruses
Environmental Triggers - Hormonal
- Other
Juvenile idiopathic arthritis
• Represents a heterogeneous group of disorders sharing the clinical
manifestation of arthritis
• It Is the most common rheumatic disease in children and one of the
more common chronic illnesses of childhood.
• The etiology and pathogenesis of JIA are largely unknown, and the
genetic component is complex, making clear distinction among
various subtypes difficult.
REVIOSION IN TERMINLOGIES

Juvenile Rheumatoid Arthritis(JRA)-ACR 1970

Juvenile Chronic Arthritis (JCA)-EULAR 1977

Juvenile Idiopathic Arthritis (JIA)- ILAR 1997

Characteristics of the ACR and ILAR
Classifications of Childhood Chronic Arthritis
Criteria for the Classification of
Juvenile Rheumatoid Arthritis
Why JIA?
• Children <16 yrs infrequently have true rheumatoid arthritis with
nodules,errosive synovitis and positive RF

• Persistent arthritis >6 weeks to exclude common causes of arthritis

like viral arthritis,trauma,acute vasculitis like HSP and kawasaki
disease
Juvenile Idiopathic Arthritis (JIA)- ILAR 1997
1. Systemic
2. Oligoarthritis
a. Persistent
b. Extended
3. Polyarthritis (RF Negative)
4. Polyarthritis (RF positive)
5. Psoriatic Arthritis
6. Enthesitis Related Arthritis
7. Undifferentiated Arthritis
a. Fits no other categories
b. Fits more than one category
Epidemiology
• The worldwide incidence of JIA ranges from 0.8-22.6 per 100,000
children per year, with prevalence ranges from 7-401 per 100,000.
• Female predominance is seen in poly and oligo articular JIA.
• Pauciarticular JIA (oligoarthritis) is the most common subtype (50-
60%), followed by polyarticular (30-35%) and systemic-onset (10-
20%).
Cont…
• The peak age at onset is between 2 and 4 yr for pauciarticular
disease.
• Age of onset has a bimodal distribution in polyarthritis, with peaks at
2-4 yr and 10-14 yr.
• SoJIA occurs throughout childhood without a peak.
 Etiology
• JIA is a complex genetic trait in which multiple genes may affect
disease susceptibility.
• Possible nongenetic triggers include
• Bacterial and viral infections (parvovirus B19, rubella, Epstein-Barr
virus),
• Enhanced immune responses to bacterial or mycobacterial heat shock
proteins,
• Abnormal reproductive hormone levels, and
• Joint trauma.
 Clinical Manifestations
• Arthritis is defined by intra-articular swelling or the presence of 2 or more
of the following signs:
Limitation in range of motion,
Tenderness or pain on motion,
 Increased heat or erythema.
Oligoarticular JIA
• Defined as involving ≤4 joints within the first 6
mo of disease onset,
• Predominantly affecting the large joints of the
lower extremities, such as the knees and
ankles.
• Accounts for 40 o 50% of JIA
• 60% ANA positive
• Persistent oligoarthritis affecting ≤4 joints
throughout the disease course
• Extended oligoarthritis affecting >4 joints
after 1st 6 mo of disease
Polyarticular JIA
• Characterized by inflammation of ≥5 joints in
both upper and lower extremities.
• 20% of all JIA
• Symmetrical joint involvement
• Large and small joints
• ANA positive in 20%
• Classified with RF positivity
• RF positive in 3% indicating early onset adult
rheumatoid arthritis ,poor prognosis.
Cont…
• Rheumatoid nodules on the extensor surfaces of the elbows and over
the Achilles tendons, although unusual, are associated with a more
severe course and almost exclusively occur in RF-positive individuals.
 Systemic-onset disease

• Arthritis in ≥1 joint with, or preceded by, fever of at least 2 wk in

duration that is documented to be daily (quotidian* ) for at east 3
days and accompanied by ≥1 of the following:
1. Evanescent (nonfixed) erythematous rash
2. Generalized lymph node enlargement
3. Hepatomegaly or splenomegaly or both
4. Serositis
• Accounts to 5-15 % of JIA
 Cont…
• The characteristic fever, defined as spiking temperatures to ≥39?C, occurs
on a daily or twice-daily basis for at least 2 wk, with a rapid return to
normal or subnormal temperatures.
• The fever is often present in the evening and is frequently accompanied
by a characteristic faint, erythematous, macular rash (salmon-colored
lesions).
Cont…
• Koebner phenomenon, a cutaneous hypersensitivity to superficial
trauma, is often present.
 Cont…
• Macrophage activation syndrome (MAS) is a rare but potentially fatal
complication of SoJIA that can occur at anytime during the disease
course.
• MAS classically manifests as acute onset of profound anemia associated
with thrombocytopenia or leukopenia with high, spiking fevers,
lymphadenopathy, and hepatosplenomegaly.
• ESR falls because of hypofibrinogenemia and hepatic dysfunction, a
feature useful in distinguishing MAS from a flare of systemic disease.
Features of Macrophage Activation Syndrome
Complications of JIA
Growth Disturbance
Bone and joint destruction
Cont…
Uveitis
•Prevalence
All subtypes : 12 to 15%
Oligoarthritis :30% of all
•Onset is With in 3 yrs of JIA diagnosis 80% and
Before arthritis in 25%
•Course
Mild intermitant:50%
Chronic refractory to
treatment:50%......blindness
•No correlation between course of arthritis and
uveitis
•ANA positivity correlated with uveitis
Differential diagnosis
Diagnosis
• JIA is a clinical diagnosis of exclusion with many mimics and without
diagnostic laboratory tests.
• Laboratory studies, including tests for ANA and RF, are only
supportive and their results may be normal in patients with JIA.
Laboratory Findings
• CBC with diff
• ESR,CRP
• Elevated ANA titers are present in 40-85% of children with
oligoarticular or 5-10% polyarticular JIA but are rare with SoJIA.
• ANA seropositivity is associated with increased risk of chronic uveitis
in JIA.
• SoJIA-
Treatment
• Goal of treatment are
Achieve disease remission,
Prevent or halt joint damage, and
Foster normal growth and development
Cont…
1. Oligoarthicular JIA
NSAIDs(partial response)
Corticosteroid (intra-articular)-for those who have no response after 4-6 wk
of treatment with NSAIDS or who have functional limitations.
Disease-modifying antirheumatic drugs (DMARDs)
Cont…
2. Polyarticular disease
NSAIDs(rarely induce remission)
DMARDs(e.g. Methotrexate)
Biologic DMARDs--TNF-α antagonists (e.g., etanercept, adalimumab,
infliximab)
Cont…
3. SoJIA
Combination of TNF-α blockade and methotrexate can be used.
Systemic CS- for severe systemic illness, for bridge therapy during the
wait for therapeutic response to a DMARD, and for control of uveitis
Supportive therapy
• Management of JIA must include periodic slit-lamp ophthalmologic
examinations to monitor for asymptomatic uveitis.
• Dietary evaluation and counseling to ensure appropriate calcium,
vitamin D, protein, and caloric intake.
• Physical therapy and occupational therapy are invaluable adjuncts to
any treatment program.
Prognosis
• Studies analyzing management of JIA in the pre-TNF-α era indicate
that up to 50% of patients with JIA have active disease persisting into
early adulthood, often with severe limitations of physical function.
• Children with persistent oligoarticular disease fare well, with a
majority achieving disease remission. Those in whom more extensive
disease develops have a poorer prognosis.
Cont…
• Children with oligoarthritis, particularly girls who are ANA positive
and with onset of arthritis earlier than 6 yr of age are at risk for
development of chronic uveitis.
Cont…
• Poor prognostic indicator for polyarticular JIA include
young age at onset,
RF seropositivity or rheumatoid nodules,
presence of anti-CCP antibodies,
large numbers of affected joints,
Disease involving the hip and hand and wrist
• Poorer prognosis for SoJIA
• Polyarticular distribution of arthritis,
• Fever lasting >3 mo, and
• Increased inflammatory markers, such as platelet count and ESR,
for >6 mo